2024/12/31 by Chioukh FZ., Ben Ameur, K., Ben Hamida, H. +5
#Aciduries organiques #Acroderma- tite entéropathica #Nouveau-né #dermatology #epider- molysis bullosa #neonate #pyloric atresia #surgery
paper · doi:10.71566/pist-rmp-194861
Background :Cutaneous lesions resembling acrodermatitis enteropathica were described in some metabolic disorders like organic aciduria caused by an enzymatic deficiency involved in the degradative pathways of branched- chain amino acids. We report two neonatal observations of acrodermatitis enteropathica-like syndrome induced by essential amino acid deficiency. Case reports :Case 1: An 8- days- old baby was referred to our hospital for neurological distress. Maple syrup urine disease was diagnosed. He was being fed a low-protein diet limited in branched-chain amino acids. One week later, exfoliative dermatitis of the perioral and anogenital regions was observed. Iatrogenic acrodermatitis ente- ropathica-like syndrome was confirmed after a rapid response after adequate isoleucine supplementation. Case 2: A newborn was referred to our intensive care unit for hyperammonemia due to methylmalonic acidemia (MMA) confirmed by metabolic screening. Cutaneous lesions resembling acrodermatitis entero- pathica appeared after 6 days of low-protein diet limited in branched-chain amino acids. An adequate supplementation of deficient amino acids led to a prompt improvement of the skin lesions. Conclusion :Skin disorders such as staphylococcal scalded-skin syndrome with epidermolysis or as acro- dermatitis enteropathica-like syndrome occur usually in the course of late and severe MSUD, propionic aciduria or MMA decompensations. In our two cases it occured early in the second week in the dietary management of neona- tal MSUD and MMA decompensation. It can be ascribed to acute and precocious protein malnutrition mostly to L-isoleucine deficiency. A better and early supplementation in L-iso- leucine could avoid such hazardous complica- tions.